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Acquired Von Willebrand Syndrome (acquired + von_willebrand_syndrome)
Selected AbstractsA new ELISA assay for diagnosis of acquired von Willebrand SyndromeHAEMOPHILIA, Issue 3 2003C. Siaka Summary. The pathophysiology of acquired von Willebrand syndrome (AVWS), a rare bleeding disorder, is not fully understood. Circulating antibodies to Von Willebrand factor (VWF) are found in patients with AVWS associated with lymphoproliferative disorders but these autoantibodies are difficult to detect with routine laboratory tests and neutralisation assays. We have developed a simple enzyme-linked immunosorbent assay (ELISA) to detect serum antibody binding to VWF protein immobilized on polystyrene plates. Ten patients with AVWS were studied, eight of whom also had lymphoproliferative disorders. We found antibodies in eight patients; all of them were positive for IgG and five were also positive for IgM. This simple method appears to be more sensitive than functional assays, which failed to identify two of the patients who were positive with the ELISA. In conjunction with other tests, this ELISA method may be useful for demonstrating the immunological mechanism underlying some cases of AVWS. Such patients would qualify for intravenous immunoglobulin therapy, which can correct the clotting disorder. [source] Polycythemia vera-associated acquired von Willebrand syndrome despite near-normal platelet count,AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2010Ayalew Tefferi No abstract is available for this article. [source] Bortezomib effectiveness in one patient with acquired von Willebrand syndrome associated to monoclonal gammopathy of undetermined significance,AMERICAN JOURNAL OF HEMATOLOGY, Issue 5 2010Mario Ojeda-Uribe No abstract is available for this article. [source] Management of acquired von Willebrand's sryndrome in a patient requiring major surgeryHAEMOPHILIA, Issue 6 2005J. M. Maddox Summary., We present the case of a patient with acquired von Willebrand's syndrome and a monoclonal gammopathy of undetermined significance who required cystectomy for relapsed transitional cell carcinoma (TCC) of the bladder. We demonstrated that infused von Willebrand factor (VWF) containing factor VIII concentrates had an unacceptably short half-life, but that this was significantly prolonged following combined therapy with plasma exchange and intravenous immunoglobulin (IVIgG). This approach was successfully utilized peri-operatively, with the total surgical blood loss less than would be expected even for a haemostatically normal patient. Trough VWF antigen and Ristocetin co-factor activity levels fell on the second postoperative day and we therefore administered further IVIgG. Levels again fell on the fifth postoperative day with the development of a Staphylococcus aureus septicaemia. At this point bleeding occurred from a surgical drain site requiring ,factor VIII inhibitor bypass activity' to secure haemostasis while further plasma exchange and IVIgG were administered. Now 5 years later, there is no evidence of recurrence of the TCC or progression of the monoclonal gammopathy. [source] | ||||||||||